Differential prognostic impact of resting heart rate in older compared with younger patients with chronic heart failure--insights from TIME-CHF.

BACKGROUND: There is little information regarding the prognostic role of resting heart rate (HR) in older compared with younger patients with chronic heart failure (HF). METHODS AND RESULTS: In patients enrolled in the Trial of Intensified Medical Therapy in Elderly Patients With Congestive Heart Failure (TIME-CHF) with sinus rhythm, effects of baseline HR (≥70 vs <70 beats/min [bpm]) on 18-month outcomes were compared between older (≥75 years; n = 186) and younger (<75 years; n = 141) patients. Older patients with lower (61 ± 6 bpm) and higher (83 ± 9 bpm) HR had similar left ventricular ejection fraction (LVEF), New York Heart Association (NYHA) functional class, N-terminal pro-B-type natriuretic peptide (NT-proBNP), and survival and HF hospitalization-free survival. In contrast, younger patients with higher HR (81 ± 7 bpm) had higher NT-proBNP and NYHA functional class, lower LVEF, and a higher risk of death (hazard ratio 4.01 [95% confidence interval (CI) 1.17 -13.69]; P = .02) and death or HF hospitalization (hazard ratio 2.35 [95% CI 1.01-5.50]; P = .04) than those with lower HR (62 ± 5 bpm), with the association between higher HR and survival remaining significant after adjustment for NYHA functional class, LVEF, and NT-proBNP. CONCLUSIONS: In contrast to HF patients aged <75 years, we found no association between HR and worse outcomes in HF patients aged ≥75 years.

Reduction of ST-elevation myocardial infarction in Canton Ticino (Switzerland) after smoking bans in enclosed public places--No Smoke Pub Study.

BACKGROUND: Second-hand smoke increases the risk of acute myocardial infarction. Canton Ticino (CT) first introduced a smoking ban in public places in 2007. This offered the opportunity to assess the long-term impact of a smoking ban on the incidence of ST-elevation myocardial infarctions (STEMI) compared with a population where the law was not yet implemented. METHODS: We assessed the incidence of STEMI hospitalizations per 100 000 inhabitants both during 3 years before and after the ban application in CT and in Canton Basel City (CBC), where this law was not yet applied. Data were obtained from the codified hospital registry (ICD-10 codes). RESULTS: In CT, the mean incidence of STEMI admissions during the 3 pre-ban years (123.7) was significantly higher than the incidence of admissions in each of the 3 post-ban years (92.9, 101.6 and 89.6 respectively; P <.024). Analysing population subsets, a post-ban reduction was observed among ≥65-year-old people of both sexes in each of the 3 post-ban years and in the <65-year age group during the first post-ban year (P = 0.02). Conversely, the mean incidence of STEMI hospitalizations in CBC (92.4) didn't change significantly in each of the 3 post-ban years (83.9, 83.3 and 79.5, P = NS) during the same period. However, a significant long-term reduction in STEMI admissions was observed in CBC among the male group with ≥65 years (P < 0.01). CONCLUSION: Our work suggests a significant impact of the smoke-free policy on the number of annual STEMI. Specific population subsets (i.e. ≥65-year-old females) were particularly affected by the smoking ban, showing a significant reduction in STEMI hospitalizations.

An in-silico analysis of the effect of heart position and orientation on the ECG morphology and vectorcardiogram parameters in patients with heart failure and intraventricular conduction defects.

AIM: The aim of this study was to investigate the influence of geometrical factors on the ECG morphology and vectorcardiogram (VCG) parameters. METHODS: Patient-tailored models based on five heart-failure patients with intraventricular conduction defects (IVCDs) were created. The heart was shifted up to 6 cm to the left, right, up, and down and rotated ±30° around the anteroposterior axis. Precordial electrodes were shifted 3 cm down. RESULTS: Geometry modifications strongly altered ECG notching/slurring and intrinsicoid deflection time. Maximum VCG parameter changes were small for QRS duration (-6% to +10%) and QRS-T angle (-6% to +3%), but considerable for QRS amplitude (-36% to +59%), QRS area (-37% to +42%), T-wave amplitude (-41% to +36%), and T-wave area (-42% to +33%). CONCLUSION: The position of the heart with respect to the electrodes is an important factor determining notching/slurring and voltage-dependent parameters and therefore must be considered for accurate diagnosis of IVCDs.

Interaction between pulmonary hypertension and diastolic dysfunction in an elderly heart failure population.

BACKGROUND: Pulmonary hypertension due to left heart disease is very common. Our aim was to investigate the relationship of the severity of left ventricular diastolic dysfunction with precapillary and postcapillary pulmonary hypertension (PH) in an elderly heart failure (HF) population. METHODS AND RESULTS: A post hoc analysis of the Trial of Intensified Medical Therapy in Elderly Patients With Congestive Heart Failure data was done. Baseline transthoracic echocardiography was used to categorize diastolic function, estimate pulmonary artery pressure and pulmonary capillary wedge pressure, and calculate the transpulmonary pressure gradient (TPG). Among 392 HF patients, PH was present in 31% of patients with grade 1, in 37% of patients with grade 2, and in 65% of patients with grade 3 diastolic dysfunction; 54% of all HF patients with PH had a TPG >12 mm Hg, suggesting not only a postcapillary but also an additional precapillary component of PH. Survival was not related to the severity of diastolic dysfunction, but was worse in patients with PH (hazard ratio 1.63, 95% confidence interval 1.07-2.51; P = .024). CONCLUSIONS: Our data indicate that HF patients with even mild diastolic dysfunction often have PH. Echocardiographic assessment suggest that the presence of PH might not simply be due to increased PCWP, but in part due to a precapillary component.

Patient-specific modelling of cardiac electrophysiology in heart-failure patients.

AIMS: Left-ventricular (LV) conduction disturbances are common in heart-failure patients and a left bundle-branch block (LBBB) electrocardiogram (ECG) type is often seen. The precise cause of this pattern is uncertain and is probably variable between patients, ranging from proximal interruption of the left bundle branch to diffuse distal conduction disease in the working myocardium. Using realistic numerical simulation methods and patient-tailored model anatomies, we investigated different hypotheses to explain the observed activation order on the LV endocardium, electrogram morphologies, and ECG features in two patients with heart failure and LBBB ECG. METHODS AND RESULTS: Ventricular electrical activity was simulated using reaction-diffusion models with patient-specific anatomies. From the simulated action potentials, ECGs and cardiac electrograms were computed by solving the bidomain equation. Model parameters such as earliest activation sites, tissue conductivity, and densities of ionic currents were tuned to reproduce the measured signals. Electrocardiogram morphology and activation order could be matched simultaneously. Local electrograms matched well at some sites, but overall the measured waveforms had deeper S-waves than the simulated waveforms. CONCLUSION: Tuning a reaction-diffusion model of the human heart to reproduce measured ECGs and electrograms is feasible and may provide insights in individual disease characteristics that cannot be obtained by other means.

Quality assessment of cardiovascular magnetic resonance in the setting of the European CMR registry: description and validation of standardized criteria.

BACKGROUND: Cardiovascular magnetic resonance (CMR) has become an important diagnostic imaging modality in cardiovascular medicine. However, insufficient image quality may compromise its diagnostic accuracy. We aimed to describe and validate standardized criteria to evaluate a) cine steady-state free precession (SSFP), b) late gadolinium enhancement (LGE), and c) stress first-pass perfusion images. These criteria will serve for quality assessment in the setting of the Euro-CMR registry. METHODS: Thirty-five qualitative criteria were defined (scores 0-3) with lower scores indicating better image quality. In addition, quantitative parameters were measured yielding 2 additional quality criteria, i.e. signal-to-noise ratio (SNR) of non-infarcted myocardium (as a measure of correct signal nulling of healthy myocardium) for LGE and % signal increase during contrast medium first-pass for perfusion images. These qualitative and quantitative criteria were assessed in a total of 90 patients (60 patients scanned at our own institution at 1.5T (n=30) and 3T (n=30) and in 30 patients randomly chosen from the Euro-CMR registry examined at 1.5T). Analyses were performed by 2 SCMR level-3 experts, 1 trained study nurse, and 1 trained medical student. RESULTS: The global quality score was 6.7±4.6 (n=90, mean of 4 observers, maximum possible score 64), range 6.4-6.9 (p=0.76 between observers). It ranged from 4.0-4.3 for 1.5T (p=0.96 between observers), from 5.9-6.9 for 3T (p=0.33 between observers), and from 8.6-10.3 for the Euro-CMR cases (p=0.40 between observers). The inter- (n=4) and intra-observer (n=2) agreement for the global quality score, i.e. the percentage of assignments to the same quality tertile ranged from 80% to 88% and from 90% to 98%, respectively. The agreement for the quantitative assessment for LGE images (scores 0-2 for SNR <2, 2-5, >5, respectively) ranged from 78-84% for the entire population, and 70-93% at 1.5T, 64-88% at 3T, and 72-90% for the Euro-CMR cases. The agreement for perfusion images (scores 0-2 for %SI increase >200%, 100%-200%,<100%, respectively) ranged from 81-91% for the entire population, and 76-100% at 1.5T, 67-96% at 3T, and 62-90% for the Euro-CMR registry cases. The intra-class correlation coefficient for the global quality score was 0.83. CONCLUSIONS: The described criteria for the assessment of CMR image quality are robust with a good inter- and intra-observer agreement. Further research is needed to define the impact of image quality on the diagnostic and prognostic yield of CMR studies.

Cardiovascular MR imaging after surgical correction of tetralogy of Fallot: approach based on understanding of surgical procedures.

Tetralogy of Fallot (TOF) is one of the most common congenital heart diseases for which patients are referred for postoperative magnetic resonance (MR) imaging evaluation. The most common surgical procedures for TOF repair include infundibulectomy, transannular pulmonary artery patch repair, and right ventricle-pulmonary artery conduit placement. In the past few decades, surgery has proved successful, but most patients require repeat imaging throughout their lives. MR imaging is now frequently used for morphologic and functional evaluation after TOF repair. The most common late postoperative sequelae and residual lesions include right ventricular outflow tract aneurysm and dyskinesis, conduit failure, pulmonary regurgitation, tricuspid regurgitation, right ventricular failure, residual main and branch pulmonary artery stenosis, branch pulmonary artery aneurysm, left pulmonary artery kinking, and residual or recurrent ventricular septal defect. The imaging approach for the evaluation of patients with repaired TOF should be guided by the surgical procedure used and the complications that are expected. Knowledge of the most common postoperative problems and their cardiovascular MR imaging appearances is essential for good radiology practice in this clinical setting.

End-of-life preferences of elderly patients with chronic heart failure.

AIMS: Elderly heart failure (HF) patients are assumed to prefer improved quality of life over longevity, but sufficient data are lacking. Therefore, we assessed the willingness to trade survival time for quality-of-life (QoL) and the preferences for resuscitation. METHODS AND RESULTS: At baseline and after 12 and 18 months, 622 HF patients aged ≥60 years (77 ± 8 years, 74% NYHA-class ≥III) participating in the Trial of Intensified vs. standard Medical therapy in Elderly patients with Congestive Heart Failure had prospective evaluation of end-of-life preferences by answering trade-off questions (willingness to accept a shorter life span in return for living without symptoms) and preferences for resuscitation if necessary. The time trade-off question was answered by 555 patients (89%), 74% of whom were not willing to trade survival time for improved QoL. This proportion increased over time (Month 12: 85%, Month 18: 87%, P < 0.001). In multivariable analysis, willingness to trade survival time increased with age, female sex, a reduced Duke Activity Status Index, Geriatric Depression Score, and history of gout, exercise intolerance, constipation and oedema, but even combining these variables did not result in reliable prediction. Of 603 (97%) patients expressing their resuscitation preference, 51% wished resuscitation, 39% did not, and 10% were undecided, with little changes over time. In 430 patients resuscitation orders were known; they differed from patients' preferences 32% of the time. End-of-life preferences were not correlated to 18-month outcome. CONCLUSION: Elderly HF patients are willing to address their end-of-life preferences. The majority prefers longevity over QoL and half wished resuscitation if necessary. Prediction of individual preferences was inaccurate.

Incidence, clinical predictors, and prognostic impact of worsening renal function in elderly patients with chronic heart failure on intensive medical therapy.

BACKGROUND: Incidence, predictors, and prognostic impact of worsening renal function (WRF) in elderly patients with chronic heart failure (HF) undergoing intensive contemporary medical therapy are unknown. METHODS AND RESULTS: In 566 patients (age 77 ± 8 years) included in the TIME-CHF, serum creatinine (sCr) was repeatedly measured up to 6 months. Worsening renal function was classified as increase in sCr by 0.2 to 0.3 (WRFI), 0.3 to 0.5 (WRFII), or ≥0.5 mg/dL (WRFIII) within the first 6 months. Outcome events were assessed for 18 months. RESULTS: The incidence of WRF I, II, and III was 12%, 19%, and 22%, respectively. Worsening renal function III was associated with increased mortality (hazard ratio 1.98 [95% CI 1.27-3.07, P = .002] vs no WRF), whereas WRF I/II was not. History of renal failure, spironolactone treatment, higher baseline dose, and higher maximal increase in loop diuretic dose were independently associated with the occurrence of WRF III, whereas angiotensin-converting enzyme inhibitor, angiotensin receptor blocker, and ß-blocker use and allocation to N-terminal pro-B-type natriuretic peptide-guided management were not. Worsening renal function III was an independent predictor of death, death or hospitalization, and death or HF hospitalization also after adjusting for baseline characteristics. CONCLUSIONS: One fifth of elderly patients with chronic HF experienced WRF III on 6-month intensive HF treatment. These patients had higher mortality, whereas patients with smaller sCr rises did not. Occurrence of WRF III was associated with high doses of loop diuretics and spironolactone use but not with other treatments.

Cost evaluation of cardiovascular magnetic resonance versus coronary angiography for the diagnostic work-up of coronary artery disease: application of the European Cardiovascular Magnetic Resonance registry data to the German, United Kingdom, Swiss, and United States health care systems.

BACKGROUND: Cardiovascular magnetic resonance (CMR) has favorable characteristics for diagnostic evaluation and risk stratification of patients with known or suspected CAD. CMR utilization in CAD detection is growing fast. However, data on its cost-effectiveness are scarce. The goal of this study is to compare the costs of two strategies for detection of significant coronary artery stenoses in patients with suspected coronary artery disease (CAD): 1) Performing CMR first to assess myocardial ischemia and/or infarct scar before referring positive patients (defined as presence of ischemia and/or infarct scar to coronary angiography (CXA) versus 2) a hypothetical CXA performed in all patients as a single test to detect CAD. METHODS: A subgroup of the European CMR pilot registry was used including 2,717 consecutive patients who underwent stress-CMR. From these patients, 21% were positive for CAD (ischemia and/or infarct scar), 73% negative, and 6% uncertain and underwent additional testing. The diagnostic costs were evaluated using invoicing costs of each test performed. Costs analysis was performed from a health care payer perspective in German, United Kingdom, Swiss, and United States health care settings. RESULTS: In the public sectors of the German, United Kingdom, and Swiss health care systems, cost savings from the CMR-driven strategy were 50%, 25% and 23%, respectively, versus outpatient CXA. If CXA was carried out as an inpatient procedure, cost savings were 46%, 50% and 48%, respectively. In the United States context, cost savings were 51% when compared with inpatient CXA, but higher for CMR by 8% versus outpatient CXA. CONCLUSION: This analysis suggests that from an economic perspective, the use of CMR should be encouraged as a management option for patients with suspected CAD.

[Cardiac magnetic resonance in acute myocarditis: a new non-invasive diagnostic gold standard?]. / IRM cardiaque: imagerie de référence dans le diagnostic de la myocardite aiguë?

Acute myocarditis was until recently one of the most difficult diagnoses in cardiology. The spectrum of signs and symptoms is very wide, the usual non-invasive tests lack specificity and the myocardial biopsy is only performed in a minority of cases to confirm the diagnosis. Due to its unique ability to directly image myocardial necrosis, fibrosis and oedema, cardiac magnetic resonance (CMR) is now considered the primary tool for noninvasive assessment of patients with suspected myocarditis. CMR is also useful for monitoring disease activity under treatment. Myocarditis has been associated with the development of dilated cardiomyopathy; CMR could play a role in the follow-up of such cases to detect the progression toward a dilatative phenotype. Precise mapping of myocardial lesions with cardiac MRI is invaluable to guide myocardial biopsy and increase its diagnostic yield by improving sensitivity.

Tetralogy of Fallot: impact of the excursion of the interventricular septum on left ventricular systolic function and fibrosis after surgical repair.

PURPOSE: To quantify the excursion of interventricular septum (IVS) in patients after repair of tetralogy of Fallot (TOF), a marker of interventricular interaction, and assess its association with left ventricular (LV) ejection fraction, LV septal wall thickening, and LV fibrosis. MATERIALS AND METHODS: The HIPAA-compliant protocol received institutional board review approval. IVS excursion was measured at cardiovascular magnetic resonance (MR) imaging in 82 patients after repair of TOF and in 10 healthy volunteers. IVS excursion was correlated with LV ejection fraction, LV septal wall thickening, and LV delayed gadolinium enhancement. Independent predictors of reduced LV ejection fraction were identified, including significant univariable predictors with use of a multivariable logistic regression model. RESULTS: IVS excursion was greater in patients than in healthy volunteers (5.3 mm ± 3.1 vs 1.2 mm ± 0.4, P < .01). Patients (n = 68) with abnormal excursion of the IVS had reduced LV ejection fraction (57% ± 7 vs 61% ± 4, P < .01) and reduced LV septal wall thickening (24% ± 10 vs 29% ± 5, P = .01) compared with patients with normal IVS excursion. Maximal IVS excursion (odds ratio = 1.27 per millimeter, P = .03) and right ventricular (RV) ejection fraction (odds ratio = 0.92 per percentage, P = .031) were independent predictors of reduced LV ejection fraction (<55%). Among the 44 patients with delayed enhancement images, those with abnormal excursion of the IVS had higher LV delayed enhancement scores (median, 1.5 [interquartile range, 0-2] vs 0 [interquartile range, 0-0]; P < .01] than patients with normal IVS excursion. Notably, in all but one patient the delayed enhancement was located at the RV-LV hinge points. CONCLUSION: Abnormal IVS excursion after repair of TOF is associated with reduced global and septal LV systolic function and LV fibrosis at the RV-LV hinge points, suggesting a mechanism of adverse interventricular interaction.

Cardiovascular MRI for the assessment of heart failure: focus on clinical management and prognosis.

Cardiovascular MR (CMR) has an emerging role in the noninvasive diagnostic assessment of heart failure (HF). Different imaging sequences allow for a detailed assessment of cardiac morphology, function, myocardial perfusion, tissue characterization, and blood flow measurement. This article reviews the key applications of CMR in HF, with special focus on how CMR may influence the diagnostic and therapeutic approach of HF patients.

Diagnostic value of the flow profile in the distal descending aorta by phase-contrast magnetic resonance for predicting severe coarctation of the aorta.

PURPOSE: To compare aortic flow profiles at the level of the proximal descending (PDAo) and distal descending aorta (DDAo) in patients investigated for coarctation of the aorta (CoA), and compare their respective diagnostic value for predicting severe CoA. Diastolic flow decay in the PDAo predicts severe CoA, but flow measurements at this level are limited by flow turbulence, aliasing, and stent-related artifacts. MATERIALS AND METHODS: We studied 49 patients evaluated for CoA with phase contrast magnetic resonance imaging (PC-MRI). Parameters of diastolic flow decay in the PDAo and DDAo were compared. Their respective diagnostic value was compared with the standard reference of transcatheter peak gradient ≥20 mmHg. RESULTS: Flow measurement in the PDAo required repeated acquisition with adjustment of encoding velocity or location of the imaging plane in 69% of patients; measurement in the DDAo was achieved in single acquisition in all cases. Parameters of diastolic flow decay in the PDAo and DDAo, including rate-corrected (RC) deceleration time and RC flow deceleration yielded a good correlation (r = 0.78; P < 0.01, and r = 0.92; P < 0.01), and a similar diagnostic value for predicting severe CoA. The highest diagnostic accuracy was achieved by RC deceleration time at DDAo (sensitivity 85%, specificity 85%). CONCLUSION: Characterization of aortic flow profiles at the DDAo offers a quick and reliable noninvasive means of assessing hemodynamically significant CoA.

Predictors of early readmission or death in elderly patients with heart failure.

BACKGROUND: Contemporary heart failure (HF) patients are elderly and have a high rate of early rehospitalization or death, resulting in a high burden for both the patients and the health care system. Prior studies were focused on younger and less well-characterized patients. We aimed to identify predictors of early hospital readmission and death in elderly patients with HF. METHODS: Patients with chronic HF taking part in the TIME-CHF study (n = 614, age 77 +/- 8 years, 41% female, left ventricular ejection fraction 35% +/- 13%) were evaluated with respect to predictors of hospital readmission or death 30 and 90 days after inclusion. Demographic, clinical, laboratory, echocardiographic, and social variables were obtained at baseline and included in a multivariable logistic regression analysis to identify predictors of early events. RESULTS: The rate of hospital readmission or death was high at 30 (11%) and 90 days (26%). The reason for hospitalization was HF in 33%, other cardiovascular in 32%, and noncardiovascular in 45% of the cases, respectively. Predictors of readmission or death at 30 days were angina, lower systolic blood pressure, anemia, more extensive edema, higher creatinine levels, and dry cough; and at 90 days were coronary artery disease, prior pacemaker implantation, high jugular venous pressure, pulmonary rales, prior abdominal surgery, older age, and depressive symptoms. CONCLUSIONS: Early hospital readmission or death was frequent among elderly HF patients. A very large proportion of readmissions were due to noncardiovascular causes. In addition to clinical signs of HF, comorbidities are important predictors of early events in elderly HF patients.

Interrelation of ST-segment depression during bicycle ergometry and extent of myocardial ischaemia by myocardial perfusion SPECT.

PURPOSE: The aim of this study was to compare ST-segment depression (STD) during bicycle ergometry and extent of myocardial ischaemia assessed by myocardial perfusion SPECT (MPS) in a large patient cohort. METHODS: Consecutive patients (n = 955) referred for MPS with bicycle ergometry and interpretable stress ECG were evaluated with respect to ECG and MPS findings of ischaemia. The maximal STD was recorded and exercise ECG was considered ischaemic if STD was horizontal or downsloping (>or=1 mm). MPS was interpreted using a 20-segment model with a scale of 0 to 4. A summed stress (SSS), summed rest (SRS) and summed difference score (SDS = SSS-SRS, e.g. extent of ischaemia) were derived. Ischaemia was defined as an SDS >or= 2. RESULTS: An exercise-induced STD was present in 215 patients (22%) and myocardial ischaemia on MPS was present in 366 patients (38%). The extent of ST-segment depression and the number of ECG leads with significant STD were each strongly and significantly associated with increasing severity of ischaemia and the number of coronary territories involved (p < 0.01 for all correlations). CONCLUSION: These data demonstrate a strong correlation between the extent of STD, number of ischaemic leads and severity of myocardial ischaemia as assessed by MPS during bicycle ergometry.

Detection of intake of nonsteroidal anti-inflammatory drugs in elderly patients with heart failure. How to ask the patient?

PRINCIPLES: Heart failure hospitalisations may be related to non-steroidal anti-inflammatory drug (NSAID) use. Since NSAIDs are usually prescribed by general practitioners or taken without prescription, their use may be largely underestimated. Therefore, we assessed the impact of a focussed analgesic medication history as compared to a usual medication history on detection of NSAID intake in elderly heart failure patients and the potential effect of medical advice on discontinuation of this therapy in a non-controlled study design. METHODS: A structured and stepwise history of analgesic intake (firstly open questioning about medication intake, secondly with a focus on analgesic intake, finally focussing on behaviour in case of pain) was done in 197 elderly heart failure patients taking part in the TIME-CHF study at baseline and up to 3 follow-up visits. All participants were informed about the potential hazardous effects of NSAIDs and alternative analgesic therapy was proposed in case of NSAID intake. Patients were aged 60 years or older with clinical signs of heart failure NYHA > or =II, elevated NT-BNP, and had been hospitalised due to heart failure within the last year. Details of this study have been described previously. RESULTS: At baseline, 43 patients (22%) were taking NSAID. Almost half (n = 19) taking NSAID reported the use only after specific questioning. Therefore, a focussed analgesic medication history was superior as compared to a usual medication history to detect patients taking NSAIDs (22% vs 12%; p <0.001). After instruction and proposal of alternative analgesic therapy, NSAID intake dropped from 22% to 7% (p <0.001). No risk factor for continuous use was identified. CONCLUSIONS: NSAID use in heart failure patients is relatively common. Specific questioning may help to increase detection of NSAID intake and information on its hazardous effects to decrease NSAID use.

Left ventricular non-compaction: is it really isolated?

Isolated ventricular non-compaction (IVNC) is a genetically determined cardiac disease mainly involving the left ventricle. Since the first description and recognition of IVNC as a distinct cardiomyopathy, many cases have been described. However, the associated right-sided cardiac anomalies are unusual. Two cases of IVNC showing associated right-sided pathology on transthoracic echocardiography are presented.